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Case Report: Ectopic Adrenocortical Carcinoma in the Ovary

Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million habitants/year. ACCs are rare and usually endocrinologically functional. We present the case of a 59-year-old woman who experienced abdominal fullness for 6 months and increased abdominal circumference. A...

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Autores principales: Tsai, Wen-Hsuan, Chen, Tze-Chien, Dai, Shuen-Han, Zeng, Yi-Hong
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8018272/
https://www.ncbi.nlm.nih.gov/pubmed/33815299
http://dx.doi.org/10.3389/fendo.2021.662377
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author Tsai, Wen-Hsuan
Chen, Tze-Chien
Dai, Shuen-Han
Zeng, Yi-Hong
author_facet Tsai, Wen-Hsuan
Chen, Tze-Chien
Dai, Shuen-Han
Zeng, Yi-Hong
author_sort Tsai, Wen-Hsuan
collection PubMed
description Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million habitants/year. ACCs are rare and usually endocrinologically functional. We present the case of a 59-year-old woman who experienced abdominal fullness for 6 months and increased abdominal circumference. A large pelvic tumor was observed. She underwent cytoreductive surgery and the pathological test results revealed local tumor necrosis and prominent lympho-vascular invasion. Neuroendocrine carcinoma was the first impression, but positivity for synaptophysin, alpha-inhibin, transcription factor enhancer 3 (TFE-3), calretinin (focal), and CD56 (focal) and high Ki-67-labeling proliferating index (>80%) confirmed the diagnosis of ectopic ACC. Ectopic primary aldosteronism could not be excluded. However, we did not perform saline infusion test or captopril test due to poor performance status. When pathological test reports reveal neuroendocrine features not typically found in the organ being examined, IHC staining should be performed to rule out ectopic ACC. Whether the ectopic ACC is functional or not requires complete survey.
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spelling pubmed-80182722021-04-03 Case Report: Ectopic Adrenocortical Carcinoma in the Ovary Tsai, Wen-Hsuan Chen, Tze-Chien Dai, Shuen-Han Zeng, Yi-Hong Front Endocrinol (Lausanne) Endocrinology Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million habitants/year. ACCs are rare and usually endocrinologically functional. We present the case of a 59-year-old woman who experienced abdominal fullness for 6 months and increased abdominal circumference. A large pelvic tumor was observed. She underwent cytoreductive surgery and the pathological test results revealed local tumor necrosis and prominent lympho-vascular invasion. Neuroendocrine carcinoma was the first impression, but positivity for synaptophysin, alpha-inhibin, transcription factor enhancer 3 (TFE-3), calretinin (focal), and CD56 (focal) and high Ki-67-labeling proliferating index (>80%) confirmed the diagnosis of ectopic ACC. Ectopic primary aldosteronism could not be excluded. However, we did not perform saline infusion test or captopril test due to poor performance status. When pathological test reports reveal neuroendocrine features not typically found in the organ being examined, IHC staining should be performed to rule out ectopic ACC. Whether the ectopic ACC is functional or not requires complete survey. Frontiers Media S.A. 2021-03-19 /pmc/articles/PMC8018272/ /pubmed/33815299 http://dx.doi.org/10.3389/fendo.2021.662377 Text en Copyright © 2021 Tsai, Chen, Dai and Zeng http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Endocrinology
Tsai, Wen-Hsuan
Chen, Tze-Chien
Dai, Shuen-Han
Zeng, Yi-Hong
Case Report: Ectopic Adrenocortical Carcinoma in the Ovary
title Case Report: Ectopic Adrenocortical Carcinoma in the Ovary
title_full Case Report: Ectopic Adrenocortical Carcinoma in the Ovary
title_fullStr Case Report: Ectopic Adrenocortical Carcinoma in the Ovary
title_full_unstemmed Case Report: Ectopic Adrenocortical Carcinoma in the Ovary
title_short Case Report: Ectopic Adrenocortical Carcinoma in the Ovary
title_sort case report: ectopic adrenocortical carcinoma in the ovary
topic Endocrinology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8018272/
https://www.ncbi.nlm.nih.gov/pubmed/33815299
http://dx.doi.org/10.3389/fendo.2021.662377
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